Additional evidence on the link between OxSt and mitochondrial dysregulation in FD comes from Tseng et al. [71] that reported a downregulation of the protein expression of the superoxide dismutase 2 (SOD2), a mitochondrial antioxidant, in FD-specific human-induced pluripotent stem cells, which was associated with increased ROS generation and enhanced intracellular Gb3 buildup. Here, SOD2 is linked to Fabry disease.